Journal
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY
Volume 31, Issue 1, Pages 88-91Publisher
SAGE PUBLICATIONS INC
DOI: 10.1177/10668969221095457
Keywords
primary pulmonary myxoid sarcoma; mesenchymal tumor; wedge resection; fluorescence in situ hybridization; EWSR1; ATF1 fusion gene
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Primary pulmonary myxoid sarcoma is a rare lung sarcoma that requires pathological findings and gene rearrangements for accurate diagnosis.
Primary pulmonary myxoid sarcoma is a rare lung sarcoma, mostly involving the central lung and harboring the EWSR1::CREB1 fusion. We report an exceptional case of primary pulmonary myxoid sarcoma arising in the peripheral lung and harboring an EWSR1::ATF1 gene fusion. A 67-year-old man presented with a solid nodule in the right lower lobe, and wedge resection was performed. Microscopically, the tumor consisted of reticular proliferation of uniform mildly atypical spindle cells within abundant myxoid stroma. Immunohistochemically, smooth muscle actin was positive but desmin was negative. Fluorescence in situ hybridization confirmed EWSR1 and ATF1 gene rearrangements. No recurrence was seen for 12 months. Pathological findings and gene rearrangements are important for the diagnosis of primary pulmonary myxoid sarcoma. Complete resection and careful observation are required.
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